FlashPath - Lung - Congenital Pulmonary Airway Malformation

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Transcript of FlashPath - Lung - Congenital Pulmonary Airway Malformation

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flashpathHazem ali

congenital pulmonary airway malformationHazem ali

clinicalRare (1 per 25,000 births)

formerly known as congenital cystic adenomatoid malformation

Most cases diagnosed Ante-natally (by U/S)

Many cases show spontaneous regression

Can present Post-natally as:Stillbirth with generalized anasarcaInfants with respiratory distress (cyanosis, grunting, tachypnea)Asymptomatic infantsMay present later in childhood with recurrent infection, cough or dyspneaSo they need close follow-up and elective surgery for persistent lesions within first year of life

clinicalShow communication with tracheobronchial tree

Five pathological subtypes (0, 1, 2, 3, 4)Most common is type 1Can be surgically corrected (good prognosis)

Most rare is type 0Incompatible for life (poorest prognosis)

Type 2 associates with 50% of cases of extra-lobar pulmonary sequestrationAlso can associate other anomalies like renal agenesis, diaphragmatic hernia, and cardiovascular anomalies

GrossMore common types are usually Cystic lesions

All these types have good prognosis EXCEPT type 2

Type 1Type 2Type 4NumberS i n g l e o r m u l t i p l e c y s t sSizeLarge(up to 10 cm)Small(up to 2 cm)Large(up to 10 cm)WallM o s t l y t h i n w a l lContentC l e a r or m u c i n LocationW i t h i n t h e l u n gCommunication with tracheobronchial treeP r e s e n t

I 5

gross

Type 1

Type 2

GrossLess common types are usually Solid lesions

Type 0Also called acinar dysplasia or dysgenesisWidespread and bilateralLungs appear small, firm, with fine nodularity on the surface

Type 3Large, firm, and bulky massCan involve entire lungCan cause mediastinal shift to opposite side and compress the other lung

Both types have poor prognosis

microscopyType 1Type 2Type 4SizeLarge cystsSmall cysts, back-to-backLarge cystsOutlineBronchial-likeBronchiolar-likeDilated and irregularAlveolar-likeLiningPseudostratified columnar ciliated epithelium, with Interspersed mucous cellsSingle cuboidal to columnar ciliated epitheliumFlattened epithelium Type I & II pneumocytesSub-epitheliumFibro-vascular stromaSmooth muscle fibersNo/rare cartilage islandsFibro-vascular stromaSmooth muscle fibersNo cartilageInterspersed alveolar ductsSkeletal muscle fibers could be seenFibro-vascular stromaOther featuresLepidic growth within cysts and adjacent lungRisk for Bronchioalveolar carcinoma Rare subtype called Rhabdomyomatous dysplasiaCan simulate Pleuropulmonary blastoma

Microscopy

Type 1

Type 2

MicroscopyType 0Disorganized bronchial-like structuresRespiratory epitheliumFibro-muscular connective tissueCartilage

Type 3Disorganized bronchiolo-alveolar structures Lined with cuboidal epithelium gland-likeSimulate fetal lung

Differential diagnosisOther cystic lung diseases:Congenital:Bronchogenic cystsCongenital pulmonary cystsPulmonary sequestration Extra-lobarCongenital lobar emphysemaAcquired:EmphysemaHealed abscessHoneycombingMixed:Cystic fibrosisLocated outside the lungHas a systemic rather than a pulmonic blood supplyDoes not communicate with the tracheobronchial treeSub-epithelial mucous glands and cartilageDoes not communicate with the tracheobronchial tree

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Thank youHazem ali